Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts.
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Authors
Furuta, Shunsuke
Chaudhry, Afzal N
Arimura, Yoshihiro
Dobashi, Hiroaki
Fujimoto, Shouichi
Homma, Sakae
Rasmussen, Niels
Publication Date
2017-02Journal Title
J Rheumatol
ISSN
0315-162X
Publisher
The Journal of Rheumatology
Volume
44
Issue
2
Pages
216-222
Language
eng
Type
Article
This Version
AM
Physical Medium
Print-Electronic
Metadata
Show full item recordCitation
Furuta, S., Chaudhry, A. N., Arimura, Y., Dobashi, H., Fujimoto, S., Homma, S., Rasmussen, N., & et al. (2017). Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts.. J Rheumatol, 44 (2), 216-222. https://doi.org/10.3899/jrheum.160005
Abstract
OBJECTIVE: There are differences in the frequencies of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis subgroups between different geographic regions, and we have reported differences in the phenotype of microscopic polyangiitis between Europe and Japan. In this retrospective observational study, we compared phenotypes and outcomes of granulomatosis with polyangiitis (GPA) between the United Kingdom and Japan. METHODS: We identified 128 UK and 82 Japanese patients with GPA diagnosed between 2000 and 2012. We evaluated baseline characteristics including ANCA status and organ involvement, treatment, patient and renal survival, and time to first relapse. RESULTS: Median age at onset was higher in Japan than in the UK (62.2 yrs vs 57.5 yrs, p < 0.01). The proportion of patients with proteinase 3 (PR3)-ANCA was lower in Japan than in the UK (61.0% vs 85.2%, p < 0.01), while the proportion of myeloperoxidase-ANCA was higher in Japan than the UK (34.1% vs 8.6%, p < 0.01). Serum creatinine at diagnosis was lower in Japan than the UK (68.1 μmol/l vs 101.0 μmol/l, p < 0.01). Respiratory involvement was more frequent in Japan than the UK (69.5% vs 40.6%, p < 0.01). In both countries, most patients received both glucocorticoids and cyclophosphamide. At 60 months the cumulative survival rates were 87.6% and 94.3% in Japan and the UK, respectively (p = 0.03). At 60 months the cumulative relapse rates were 37.1% and 68.1% in Japan and the UK, respectively (p < 0.01). CONCLUSION: Japanese patients with GPA were older at disease onset, with less PR3-ANCA positivity, milder renal dysfunction, and more frequent respiratory involvement than UK patients. The relapse-free survival rate was higher in Japan than the United Kingdom.
Keywords
Humans, Recurrence, Antibodies, Antineutrophil Cytoplasmic, Glucocorticoids, Prognosis, Disease-Free Survival, Treatment Outcome, Survival Rate, Retrospective Studies, Age of Onset, Phenotype, Adult, Aged, Middle Aged, Japan, Female, Male, Granulomatosis with Polyangiitis, United Kingdom
Identifiers
External DOI: https://doi.org/10.3899/jrheum.160005
This record's URL: https://www.repository.cam.ac.uk/handle/1810/302818
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